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Angiomyolipoma

MedGen UID:
64622
•Concept ID:
C0206633
•
Neoplastic Process
Synonym: Angiomyolipomas
SNOMED CT: Angiomyolipoma (19929002); Angiomyolipoma (1293131004)
 
Monarch Initiative: MONDO:0002603

Definition

A neoplasm with perivascular epithelioid cell differentiation often associated with tuberous sclerosis. It is characterized by a mixture of epithelioid cells, smooth muscle, vessels, and mature adipose tissue. The kidney is the most common site of involvement. Other sites of involvement include the liver, lung, lymph nodes, and retroperitoneum. The vast majority of cases follow a benign clinical course. However, cases of metastatic angiomyolipomas with sarcomatoid features have been described. [from NCI]

Professional guidelines

PubMed

Recommendations for the diagnosis and management of LAM: Looking towards the future.
Gupta N, McCarthy C
Respir Med Res 2023 Jun;83:101016. Epub 2023 Apr 21 doi: 10.1016/j.resmer.2023.101016. PMID: 37087907
French recommendations for the diagnosis and management of lymphangioleiomyomatosis.
Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K; OrphaLung network
Respir Med Res 2023 Jun;83:101010. Epub 2023 Mar 24 doi: 10.1016/j.resmer.2023.101010. PMID: 37087906
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Soft Tissue Perivascular Epithelioid Cell Tumors.
Hammer PM, Tan SY
Surg Pathol Clin 2024 Mar;17(1):105-118. Epub 2023 Aug 1 doi: 10.1016/j.path.2023.06.002. PMID: 38278600
Renal angiomyolipoma: from imaging to intervention.
Almazedi B, Stubbs C
Clin Radiol 2024 Jan;79(1):25-32. Epub 2023 Oct 14 doi: 10.1016/j.crad.2023.09.028. PMID: 37925365
Renal angiomyolipoma.
Çalışkan S, Gümrükçü G, Özsoy E, Topaktas R, Öztürk Mİ
Rev Assoc Med Bras (1992) 2019 Aug 5;65(7):977-981. doi: 10.1590/1806-9282.65.7.977. PMID: 31389508
Renal manifestation of tuberous sclerosis complex.
Bissler JJ, Christopher Kingswood J
Am J Med Genet C Semin Med Genet 2018 Sep;178(3):338-347. Epub 2018 Oct 11 doi: 10.1002/ajmg.c.31654. PMID: 30307110
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197

Diagnosis

Renal angiomyolipoma.
Çalışkan S, Gümrükçü G, Özsoy E, Topaktas R, Öztürk Mİ
Rev Assoc Med Bras (1992) 2019 Aug 5;65(7):977-981. doi: 10.1590/1806-9282.65.7.977. PMID: 31389508
Angiomyolipoma With Epithelial Cysts.
LeRoy MA, Rao P
Arch Pathol Lab Med 2016 Jun;140(6):594-7. doi: 10.5858/arpa.2015-0170-RS. PMID: 27232352
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197
Renal angiomyolipoma.
Lienert AR, Nicol D
BJU Int 2012 Dec;110 Suppl 4:25-7. doi: 10.1111/j.1464-410X.2012.11618.x. PMID: 23194120
Angiomyolipoma of kidney.
Eble JN
Semin Diagn Pathol 1998 Feb;15(1):21-40. PMID: 9503504

Therapy

Lung transplantation for lymphangioleiomyomatosis.
Warrior K, Dilling DF
J Heart Lung Transplant 2023 Jan;42(1):40-52. Epub 2022 Oct 8 doi: 10.1016/j.healun.2022.09.021. PMID: 36334961
mTOR Inhibition and Kidney Diseases.
Ma MKM, Yung S, Chan TM
Transplantation 2018 Feb;102(2S Suppl 1):S32-S40. doi: 10.1097/TP.0000000000001729. PMID: 29369972
Lymphangioleiomyomatosis.
Johnson SR, Taveira-DaSilva AM, Moss J
Clin Chest Med 2016 Sep;37(3):389-403. doi: 10.1016/j.ccm.2016.04.002. PMID: 27514586
Tuberous sclerosis complex.
Henske EP, Jóźwiak S, Kingswood JC, Sampson JR, Thiele EA
Nat Rev Dis Primers 2016 May 26;2:16035. doi: 10.1038/nrdp.2016.35. PMID: 27226234
Renal embolization.
Grenier N, Petitpierre F, Le Bras Y, Lasserre AS, Cornelis F
Nephrol Ther 2016 Apr;12 Suppl 1:S139-43. Epub 2016 Mar 8 doi: 10.1016/j.nephro.2016.01.009. PMID: 26968474

Prognosis

Uterine angiomyolipoma: a clinical analysis of 8 cases and literature review.
Wang J, Yang Q, Zhang N, Wang D
Arch Gynecol Obstet 2021 Jul;304(1):171-177. Epub 2021 Feb 10 doi: 10.1007/s00404-021-05992-1. PMID: 33569612
Angiomyolipoma With Epithelial Cysts.
LeRoy MA, Rao P
Arch Pathol Lab Med 2016 Jun;140(6):594-7. doi: 10.5858/arpa.2015-0170-RS. PMID: 27232352
Tuberous sclerosis complex: the past and the future.
De Waele L, Lagae L, Mekahli D
Pediatr Nephrol 2015 Oct;30(10):1771-80. Epub 2014 Dec 23 doi: 10.1007/s00467-014-3027-9. PMID: 25533384
Lymphangioleiomyomatosis.
Kelly J, Moss J
Am J Med Sci 2001 Jan;321(1):17-25. doi: 10.1097/00000441-200101000-00004. PMID: 11202475
Angiomyolipoma of kidney.
Eble JN
Semin Diagn Pathol 1998 Feb;15(1):21-40. PMID: 9503504

Clinical prediction guides

Radiologist's Disease: Imaging for Renal Cancer.
Chung A, Raman SS
Urol Clin North Am 2023 May;50(2):161-180. doi: 10.1016/j.ucl.2023.01.006. PMID: 36948664
GPNMB expression identifies TSC1/2/mTOR-associated and MiT family translocation-driven renal neoplasms.
Salles DC, Asrani K, Woo J, Vidotto T, Liu HB, Vidal I, Matoso A, Netto GJ, Argani P, Lotan TL
J Pathol 2022 Jun;257(2):158-171. Epub 2022 Mar 29 doi: 10.1002/path.5875. PMID: 35072947Free PMC Article
Uterine angiomyolipoma: a clinical analysis of 8 cases and literature review.
Wang J, Yang Q, Zhang N, Wang D
Arch Gynecol Obstet 2021 Jul;304(1):171-177. Epub 2021 Feb 10 doi: 10.1007/s00404-021-05992-1. PMID: 33569612
Lymphangioleiomyomatosis.
Doubková M, Štefániková M, Čan V, Merta Z, Svoboda M
Klin Onkol 2019 Fall;32(5):367-374. doi: 10.14735/amko2019367. PMID: 31610670
mTOR Inhibition and Kidney Diseases.
Ma MKM, Yung S, Chan TM
Transplantation 2018 Feb;102(2S Suppl 1):S32-S40. doi: 10.1097/TP.0000000000001729. PMID: 29369972

Recent systematic reviews

Rapamycin and rapalogs for tuberous sclerosis complex.
Sasongko TH, Kademane K, Chai Soon Hou S, Jocelyn TXY, Zabidi-Hussin Z
Cochrane Database Syst Rev 2023 Jul 11;7(7):CD011272. doi: 10.1002/14651858.CD011272.pub3. PMID: 37432030Free PMC Article
The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis.
Wang Q, Luo M, Xiang B, Chen S, Ji Y
Respir Res 2020 Feb 14;21(1):55. doi: 10.1186/s12931-020-1316-3. PMID: 32059669Free PMC Article
Management of hepatic angiomyolipoma: A systematic review.
Klompenhouwer AJ, Verver D, Janki S, Bramer WM, Doukas M, Dwarkasing RS, de Man RA, IJzermans JNM
Liver Int 2017 Sep;37(9):1272-1280. Epub 2017 Mar 6 doi: 10.1111/liv.13381. PMID: 28177188
Transarterial Embolization of Angiomyolipoma: A Systematic Review.
Murray TE, Doyle F, Lee M
J Urol 2015 Sep;194(3):635-9. Epub 2015 Apr 25 doi: 10.1016/j.juro.2015.04.081. PMID: 25916674
Gender differences in benign renal masses.
Mauermann J, de Martino M, Waldert M, Haitel A, Klingler HC, Remzi M, Klatte T
World J Urol 2013 Oct;31(5):1051-7. Epub 2013 Feb 16 doi: 10.1007/s00345-013-1032-4. PMID: 23417319

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Reviews

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